Macular Corneal Dystrophy in an Adolescent Managed with Deep Anterior Lamellar Keratoplasty.

INTRODUCTION: Macular corneal dystrophy (MCD) is a rare autosomal recessive stromal dystrophy. The prevalence of inherited diseases varies in different communities. It is not commonly observed in Nepal. CASE: A 14-year male presented with bilateral gradual and painless blurred vision with photophobia of three years duration. Cornea of both eyes showed focal grayish opacities in anterior and mid stroma with intervening stromal haze. There was no history of consanguinity in the family. Anterior segment ocular coherence tomography showed thinning of cornea and deposits in corneal stroma. Macular corneal dystrophy was the clinical diagnosis and the patient underwent deep anterior lamellar keratoplasty in the right eye. Histopathological examination and histochemical staining with Alcian Blue supported the diagnosis. CONCLUSION: Significant improvement in vision was observed in the operated eye. MCD may be present in communities without evidence of consanguinity. It can cause visual impairment at an early age. Timely intervention improves vision and quality of life.

X-linked Juvenile Retinoschisis in a Young Female.

X-linked juvenile retinoschisis has recessive inheritance which occurs due to RS1 gene mutation. We report an instance in a female managed with systemic and topical carbonic-anhydrase inhibitors. 18-year female presented with bilateral blurred vision for two years. Best corrected vision was 6/24 right eye and 6/12 left eye. Fundus examination, ocular coherence tomography and fundus fluorescein angiography supported the diagnosis. Systemic and topical carbonic-anhydrase inhibitors were advised and followed for six months with scrutinization of possible adverse drug reaction. Juvenile retinoschisis being rare among females, prompt diagnosis and management helps for the restoration of the vision and foveal anatomy. Keywords: Carbonic anhydrase inhibitors; female; retinoschisin ; X linked juvenile retinoschisis.

Factors Affecting the Outcome of Scleral Buckling Surgery for Primary Rhegmatogenous Retinal Detachment.

INTRODUCTION: Scleral buckle surgery retains a special place in treatment of retinal detachment despite development of new and advanced vitreoretinal surgical techniques. The outcome of any retinal detachment surgery depends on correct selection of patient, type and nature of detachment, and the expertise. This study aims to evaluate various other parameters that determine the outcome of scleral buckle surgery. METHOD: Records of 55 patients with primary rhegmatogenous retinal detachment treated with scleral buckling over a duration of 18 months that had a minimum of 3-month follow-up were retrospectively reviewed. Preoperative and postoperative characteristics were recorded. Parameters that were evaluated to determine the outcome were best-corrected visual acuity (BCVA), anatomical success, and complications. RESULTS: A total of 51 eyes of 50 patients that met the inclusion criteria were included. Mean age was 41 ± 19.9 years (range: 9 to 83). Primary anatomical success was achieved in 80.4%. Parameters significantly associated with the anatomical outcome of surgery were status of lens, preoperative visual acuity, and extent of retinal detachment. There was a significant improvement of visual acuity postoperatively. CONCLUSION: Scleral buckle surgery is a highly effective surgery in uncomplicated retinal detachment cases, and single surgery success rates are better in cases with good preoperative visual acuity, partial detachment, and clear crystalline lens.